ABSTRACT
BACKGROUND: Tracheomalacia occurs as a primary developmental defect or may be secondary to vascular compression. It is common in association with esophageal atresia and tracheoesophageal fistula. Collapse of the weak trachea is a cause of recurrent respiratory symptoms but may be severe and life threatening. METHODS: Between 1978 and 1999 at Sheffield Children's Hospital and The Royal Manchester Children's Hospital, of 16 children with clinically significant symptoms of tracheomalacia 8 underwent combined aortopexy and tracheopexy, 1 had aortopexy alone, 4 only had a tracheopexy, and 3 had tracheal reinforcement with free costal cartilage ring grafts. The surgical approach was limited to a low cervical skin crease incision with a midline manubrial split providing extrapleural access to the anterior mediastinum and allowing for all surgery under direct unimpaired vision. RESULTS: Ten children did not require postoperative ventilatory support. Four underwent ventilation for a few hours or days. One child required CPAP for 4 months for residual tracheomalacia and a further child, who had 3 operations to insert 11 costal cartilage ring grafts, underwent ventilation intermittently for 6 months. Adequate tracheal patency could be verified by intraoperative tracheoscopy and was sustained postoperatively so that only 1 child with associated bilateral vocal cord paralysis came to tracheostomy. Four children required prolonged hospitalization because of residual tracheomalacia, 2 for bronchomalacia and 2 because of esophageal narrowing leading to further surgery. All other children were fit for discharge within 10 to 30 days of surgery. Long-term follow-up has confirmed sustained tracheal improvement and resolution of the life-threatening features of tracheomalacia. CONCLUSIONS: The authors recommend the low skin crease transmanubrial approach, as described by Vaishnav and MacKinnon, for tracheopexy, aortopexy and for tracheal reconstruction for tracheomalacia. The approach gives excellent access for surgery under direct vision through a relatively avascular plane. It is associated with less morbidity than a conventional thoracotomy and leaves a more acceptable aesthetic scar.
Subject(s)
Surgical Procedures, Operative/methods , Tracheal Diseases/surgery , Child, Preschool , Esophageal Atresia/complications , Follow-Up Studies , Humans , Infant , Laryngeal Cartilages/surgery , Mediastinum , Ribs , Tracheal Diseases/complications , Tracheal Diseases/diagnosis , Tracheoesophageal Fistula/complicationsABSTRACT
The authors report a rare case of persistent mullerian duct syndrome (PMDS) with transverse testicular ectopia and inguinal hernia in a 2-year-old child with family history for this syndrome. At operation the observation of a very long and thin gubernaculum and extreme mobility of both testes and uterus, which are located in the same hernial sac, allowed the Authors to propose a hypothesis to explain the role of MIF (Mullerian Inhibiting Factor) in testicular descent. Patients with PMDS present a normal outgrowth and migration phases of the gubernaculum but lack of the gubernacular regression phase. These data suggest an important function of the MIF in this phase of testicular descent.
Subject(s)
Abnormalities, Multiple/diagnosis , Cryptorchidism/diagnosis , Glycoproteins , Growth Inhibitors/physiology , Mullerian Ducts/abnormalities , Testicular Hormones/physiology , Abnormalities, Multiple/etiology , Abnormalities, Multiple/surgery , Anti-Mullerian Hormone , Child, Preschool , Cryptorchidism/etiology , Cryptorchidism/surgery , Hernia, Inguinal/diagnosis , Hernia, Inguinal/surgery , Humans , Male , Mullerian Ducts/surgery , Syndrome , Testis/surgeryABSTRACT
Las capacidades musicales fueron evaluadas en 20 pacientes afásicos, con lesiones únicas en hemisferio cerebral izquierdo. Las alteraciones musicales fueron raras en afasias de Broca (n=7), solo el 14% tenía trastornos rítmicos y el 28% melódicos. La mitad de los pacientes con afasias de Wernicke (n=4) mostraron compromiso en los aspectos rítmicos y de discriminación auditiva. Las mayores alteraciones neurosonoromusicales aparecieron en afasias globales (n=6), estando comprometidos los aspectos rítmicos en el 83% de los casos. Estas lesiones en hemisferio izquierdo mostraron que la función más alterada fue la rítmica (55%) cuyas superposiciones lesionales se encontraron sistemáticamente en el área postrolándica parietotemporal medial. Sólo el 20% tenía falla en los aspectos melódicos y el 25% en la discriminación auditiva
